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Congenital and childhood myeloproliferative disorders with eosinophilia responsive to imatinib

Shirley Abraham1, Mohamed Salama, Jeffrey Hancock

  • 1Pediatric Hematology Oncology, Department of Pediatrics, University of New Mexico, Albuquerque, New Mexico 87131-0001, USA. smabraham@salud.unm.edu

Pediatric Blood & Cancer
|April 11, 2012
PubMed

Insights

Eosinophilia in myeloproliferative disorders (MPD) with PDGFRB rearrangements responded well to imatinib treatment. Patients achieved remission, suggesting imatinib

Area of Science:

  • Hematology
  • Oncology
  • Genetics

Background:

  • Eosinophilia is a characteristic of certain myeloproliferative disorders (MPD).
  • Rearrangements in the platelet-derived growth factor receptor beta (PDGFRB) gene are implicated in a subset of MPDs.
  • Imatinib mesylate is an established treatment for some MPDs.

Observation:

  • Two patients with MPD, eosinophilia, and PDGFRB rearrangements were identified.
  • One patient had a congenital PDGFRB rearrangement.
  • Both patients received single-agent imatinib therapy.

Findings:

  • Both patients achieved clinical, hematologic, and cytogenetic remission.
  • Remission was sustained even with dose-weaning of imatinib.
  • The study highlights successful treatment outcomes in this specific MPD subset.

Implications:

  • Imatinib is a viable therapeutic option for MPDs involving PDGFRB rearrangements and eosinophilia.
  • Further research is needed to establish optimal dosing and duration guidelines for imatinib in these cases.
  • This case series may inform future treatment strategies for similar MPD presentations.

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